Results 141 to 150 of about 9,196 (170)
Some of the next articles are maybe not open access.
Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders
Nature, 2003The expansion of a CAG repeat coding for polyglutamine in otherwise unrelated gene products is central to eight neurodegenerative disorders including Huntington's disease. It has been well documented that expanded polyglutamine fragments, cleaved from their respective full-length proteins, form microscopically visible aggregates in affected individuals
Ivelisse Sanchez +2 more
exaly +3 more sources
Modulation of Molecular Chaperones in Huntington’s Disease and Other Polyglutamine Disorders [PDF]
Molecular Neurobiology, 2016 Polyglutamine expansion mutations in specific proteins underlie the pathogenesis of a group of progressive neurodegenerative disorders, including Huntington's disease, spinal and bulbar muscular atrophy, dentatorubral-pallidoluysian atrophy, and several spinocerebellar ataxias.
Sara D Reis +2 more
exaly +3 more sources
Targeting protein aggregation in neurodegeneration – lessons from polyglutamine disorders
Expert Opinion on Therapeutic Targets, 2006Polyglutamine diseases, such as Huntington's disease, are among the most common inherited neurodegenerative disorders. They share salient clinical and pathological features with major sporadic neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease and amyotropic lateral sclerosis.
Patrick Weydt
exaly +3 more sources
Histone deacetylase inhibitors as therapeutics for polyglutamine disorders
Nature Reviews Neuroscience, 2006During the past 5 years, gene expression studies in cell culture, animal models and in the brains of patients have shown that the perturbation of transcription frequently results in neuronal dysfunction in polyglutamine repeat diseases such as Huntington's disease.
Butler, R, Bates, GP
openaire +3 more sources
New light on polyglutamine neurodegenerative disorders: interference with transcription
Trends in Molecular Medicine, 2001Abstract There is accumulating evidence that aberrant transcriptional regulation is involved in neurodegenerative disorders associated with expanded polyglutamines. Recent work on the coactivator CREB-binding protein suggests that transcription factors containing a normal polyglutamine stretch might be redistributed to nuclear inclusions and ...
Christian Neri
exaly +2 more sources
Modelling of human polyglutamine neurological disorders in Drosophila
2022Polyglutamine (PolyQ) expansion diseases are a family of autosomal dominant neurodegenerative disorders that includes Huntington’s disease and spinocerebellar ataxias. These diseases are caused by an abnormal number of glutamines repeats in the affected proteins. Different in vitro and in vivo models have been developed to study these diseases; in this
Marcela CARDENAS-TUEME +3 more
openaire +1 more source
Polyglutamine diseases: a transcription disorder?
Cellular and Molecular Life Sciences (CMLS), 2003Various molecular processes including unfolded protein response, protein transport, synaptic transmission and transcription are implicated in the pathology of polyglutamine diseases caused by the expanded polyglutamine-containing proteins. More than 20 transcription-related factors have been reported to interact with disease proteins, and the ...
openaire +2 more sources
Polyglutamine disorders: Pathogenesis and potential drug interventions
Life SciencesPolyglutamine/poly(Q) diseases are a group nine hereditary neurodegenerative disorders caused due to abnormally expanded stretches of CAG trinucleotide in functionally distinct genes. All human poly(Q) diseases are characterized by the formation of microscopically discernable poly(Q) positive aggregates, the inclusion bodies.
Shweta, Tandon +2 more
openaire +2 more sources
Mini-Reviews in Medicinal Chemistry, 2007
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) tract in the huntingtin protein, resulting in intracellular aggregate formation and neurodegeneration. Biochemical pathways leading from polyQ expansion to disease pathogenesis are largely unknown.
Yoko, Kimura +2 more
openaire +2 more sources
Huntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) tract in the huntingtin protein, resulting in intracellular aggregate formation and neurodegeneration. Biochemical pathways leading from polyQ expansion to disease pathogenesis are largely unknown.
Yoko, Kimura +2 more
openaire +2 more sources
Chaperones and Polyglutamine Expansion Disorders
2008Polyglutamine expansion disorders are caused by the misfolding of proteins with abnormally long polyglutamine regions. This misfolding produces toxicity and leads to the dysfunction and ultimately to the demise of neurons in affected individuals. The molecular basis for polyglutamine toxicity is unclear and the number and complexity of documented ...
openaire +1 more source